Basics of biliary atresia to medical students.

1. BILIARY ATRESIA
Sameh Shehata

2. BILIARY ATRESIA
Progressive obliterative
cholangiopathy
incidence 1:10,000 to 1:15,000
Girls > boys

3. Etiology
1. Reovirus 3 infection.
2. Congenital malformation.
3. Congenital cytomegalovirus (CMV).
4. Autoimmune
5. Genetic

4. Types

5. ASSOCIATED ANOMALIES
Associated anomalies in 10 to 20 % of cases.
 Polysplenia syndrome:
− Cardiac malformations
− Polysplenia
− Situs inversus
− Absent vena cava
− A preduodenal portal vein
-- Bilobed symmetric liver
-- Bilobed lungs
BASM
Syndrome

6. Direct hyperbilirubinemia

8. Main DD

9. Investigations
• Liver function tests.
• US
• HIDA Isotope study.
• Percutaneous liver biopsy.
• Cholangiography.

10. Biliary USG
to exclude other etiologies like Choledochal cyst
GB absent / contracted / distended.
Triangular cord sign: Presence of fibrous cone of bile
duct remnant at porta hepatis

11. Triangular Cord Sign
Triangular Cord Sign Micky Mouse Sign

12. ISOTOPE SCAN HIDA

13. Normal uptake / No excretion

14. Poor uptake / Good excretion
NNT hepatitis

15. PERCUTANEOUS CHOLANGIOGRAPHY

16. Biliary atresia

17. (showing absent intrahepatic ducts /patent distal bile ducts

18. normal biliary tree with contrast draining

20. Percutaneous liver biopsy:
• bands of fibrous tissue
• bile duct proliferation.
• Giant cells.

21. Kasai ( porto-enterostomy)
Before 60 days

23. Post Kasai
1/3rd of pts remain asymptomatic No transplant
1/3 never have bile flow and require Early transplant
1/3 initially have good bile flow but subsequently
develop cirrhosis Late transplant

24. 2/3 will need: Liver Tx

25. Prognosis
Without surgery or liver transplant life span – 19
months
Death is due to liver failure, bleeding esophageal
varices and sepsis

26. Biliary Atresia - Mindmap